Pediatric epidermolysis bullosa acquisita: A review

Emma Hignett1, Naveed Sami2

  • 1University of Central Florida College of Medicine, Orlando, FL, USA.

Pediatric Dermatology
|August 2, 2021
PubMed

Insights

Pediatric Epidermolysis Bullosa Acquisita (EBA) is a rare autoimmune blistering disease. Reviewing 40 cases shows mucosal involvement and a favorable prognosis with treatments like corticosteroids and dapsone.

Area of Science:

  • Dermatology
  • Autoimmune Diseases
  • Pediatrics

Background:

  • Epidermolysis bullosa acquisita (EBA) is a rare acquired autoimmune blistering skin disorder.
  • Childhood EBA is exceptionally rare, necessitating focused clinical review.

Purpose of the Study:

  • To identify and analyze cases of pediatric EBA.
  • To report on the clinical features, treatment, and course of childhood EBA.

Main Methods:

  • A comprehensive literature review was performed using MEDLINE®.
  • Search terms focused on juvenile epidermolysis bullosa acquisita.

Main Results:

  • Forty cases of pediatric EBA were identified.
  • Mucosal tissues were affected in 29 out of 40 cases.
  • Treatment commonly involved systemic corticosteroids combined with dapsone, with a favorable prognosis in most cases.

Conclusions:

  • Childhood EBA, though rare, requires consideration in the differential diagnosis of pediatric blistering diseases.
  • Effective management strategies exist, leading to remission or control in the majority of identified cases.

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